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8.7: Narcolepsy and Hypersomnolence

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    Learning Objectives
    • Describe symptoms and factors associated with narcolepsy
    • Describe symptoms and factors associated with hypersomnolence disorder

    Narcolepsy

    A person falling asleep while sitting on a chair at work.
    Figure \(\PageIndex{1}\): Those with narcolepsy may have a difficult time staying awake at work.

    A person with narcolepsy cannot resist falling asleep at inopportune times. These sleep episodes are often associated with cataplexy, which is a lack of muscle tone or muscle weakness, and in some cases involves complete paralysis of the voluntary muscles similar to the kind of paralysis experienced by healthy individuals during REM sleep (Burgess & Scammell, 2012; Hishikawa & Shimizu, 1995; Luppi et al., 2011). Narcoleptic episodes take on other features of REM sleep. For example, around one-third of individuals diagnosed with narcolepsy experience vivid, dream-like hallucinations during narcoleptic attacks (Chokroverty, 2010).

    Surprisingly, narcoleptic episodes are often triggered by states of heightened arousal or stress. The typical episode can last from a minute or two to half an hour. Once awakened from a narcoleptic attack, people report that they feel refreshed (Chokroverty, 2010). Obviously, regular narcoleptic episodes could interfere with the ability to perform one’s job or complete schoolwork, and in some situations, narcolepsy can result in significant harm and injury (e.g., driving a car or operating machinery or other potentially dangerous equipment).

    In order to make a diagnosis of narcolepsy, an individual must have symptoms occurring at least three times a week over the past three months. In addition, one of the following must be present:

    • hypocretin deficiency
    • episodes of cataplexy occurring at least several times a month
    • REM sleep latency of fewer than 15 minutes or two or more sleep-onset REM periods (SOREMPs) and a mean sleep latency of fewer than eight minutes.

    Etiology

    Narcolepsy affects both males and females equally. Symptoms often start in childhood, adolescence, or young adulthood (ages seven to 25), but can occur at any time in life. It is estimated that anywhere from 135,000 to 200,000 people in the United States have narcolepsy. However, since this condition often goes undiagnosed, the number may be higher. Since people with narcolepsy are often misdiagnosed with other conditions, such as psychiatric disorders or emotional problems, it can take years for someone to get the proper diagnosis. There are two types: narcolepsy type 1 (formerly narcolepsy with cataplexy) and narcolepsy type 2 (formerly narcolepsy without cataplexy).

    Narcolepsy may have several causes. Nearly all people with narcolepsy who have cataplexy have extremely low levels of the naturally occurring chemical hypocretin, which promotes wakefulness and regulates REM sleep. Hypocretin levels are usually normal in people who have narcolepsy without cataplexy. Although the cause of narcolepsy is not completely understood, current research suggests that narcolepsy may be the result of a combination of factors working together to cause a lack of hypocretin. These factors include

    • autoimmune disorders. When cataplexy is present, the cause is most often the loss of brain cells that produce hypocretin. Although the reason for this cell loss is unknown, it appears to be linked to abnormalities in the immune system. Autoimmune disorders occur when the body’s immune system turns against itself and mistakenly attacks healthy cells or tissue. Researchers believe that in individuals with narcolepsy, the body’s immune system selectively attacks the hypocretin-containing brain cells because of a combination of genetic and environmental factors.
    • family history. Most cases of narcolepsy are sporadic, meaning the disorder occurs in individuals with no known family history. However, clusters in families sometimes occur—up to 10% of individuals diagnosed with narcolepsy with cataplexy report having a close relative with similar symptoms.
    • brain injuries. Rarely, narcolepsy results from traumatic injury to parts of the brain that regulate wakefulness and REM sleep or from tumors and other diseases in the same regions.

    Treatment

    Although there is no cure for narcolepsy, some of the symptoms can be treated with medicines and lifestyle changes. When cataplexy is present, the loss of hypocretin is believed to be irreversible and lifelong. Excessive daytime sleepiness (EDS) and cataplexy can be controlled in most individuals with medications.

    Watch It

    Watch this video about Michelle, who suffered from narcolepsy when she experienced emotional distress. She describes her symptoms with sleep disturbances, her diagnosis, and how she was empowered by her diagnosis.

    You can view the transcript for “What Is Narcolepsy?” here (opens in new window).

    Key Takeaways: Narcolepsy
    Query \(\PageIndex{1}\)
    Watch It

    This short clip explains hypersomnia.

    You can view the transcript for “What is hypersomnia?” here (opens in new window).

    Key Takeaways: Hypersomnia
    Query \(\PageIndex{1}\)
    Try It

    Insufficient Sleep Syndrome

    Although not yet classified as a disorder in the DSM-5, insufficient sleep syndrome is recognized by the International Classification of Sleep Disorders (ICSD-3) as a curtailed sleep pattern that has persisted for at least three months for most days of the week, along with complaints of sleepiness during the day. Further, a resolution of sleepiness complaints is shown to follow an extension of total sleep time. Frequently occurring episodes of insufficient sleep are associated with the experience of unfavorable mental and physical well-being. Sleep insufficiency is sometimes confused with insomnia, but the opportunity to sleep differs in the two disorders (with insomnia sufferers typically being unable to sleep despite having opportunities to do so). Insufficient sleep is associated with a range of negative health and social outcomes, including an adverse performance at school and in the labor market. Reduced sleep duration has been linked to seven of the 15 leading causes of death in the United States, including cardiovascular disease, malignant neoplasm, cerebrovascular disease, accidents, diabetes, septicemia, and hypertension. The evidence suggests that the link between inadequate sleep and negative outcomes is more direct, whereas the link between excessive sleep and negative outcomes seems to be more indirect (i.e., excessive sleep is driven by underlying chronic health conditions and not vice versa). Hence, the impact of insufficient sleep appears to be the more salient issue in our society and, because of its broad-ranging effects, represents a major public health concern. In this review, we note some of the more serious consequences of insufficient sleep, and additionally consider how these might be best addressed by changes in individual behavior, actions by employers, and by public policy measures.[footnote]Chattu, V.K.; Manzar, M.D.; Kumary, S.; Burman, D.; Spence, D.W.; Pandi-Perumal, S.R. The Global Problem of Insufficient Sleep and Its Serious Public Health Implications. Healthcare 2019, 7, 1. ↵

    Glossary

    cataplexy: lack of muscle tone or muscle weakness, and in some cases complete paralysis of the voluntary muscles

    narcolepsy: sleep disorder in which the sufferer cannot resist falling to sleep at inopportune times

    1. Since the patients’ attention levels are impaired, their quality of life may be impacted as well. This is especially true for people whose jobs request high levels of attention, for example, in the healthcare field. Indeed, the lack of attention can cause injuries to self or others, which makes this disorder a real public health issue. Hypersomnolence disorder is characterized by excessive daytime somnolence (EDS). However, the patient in question must have no other symptoms or signs of narcolepsy (cataplexy, sleep paralysis, hypnagogic hallucinations), and no breathing-related sleep disorder like sleep apnea. In addition, their excessive daytime somnolence (EDS) is not a result of insufficient sleep or due to the effects of a substance or another general medical condition. In other words, this is a diagnosis of exclusion once all other causes of excessive daytime somnolence (EDS) have been ruled out. According to ICD-10, the EDS should occur nearly every day for a month or recurrently for shorter periods of time. According to the DSM-5, the main symptom of hypersomnia is EDS, or prolonged night-time sleep, which has occurred for at least three months prior to diagnosis. Sleep drunkenness, a difficulty to transition from wake to sleep is also a symptom found in hypersomniac patients. Patients suffering from sleep drunkenness report waking with confusion, disorientation, slowness and repeated returns to sleep. According to the American Academy of Sleep Medicine, hypersomniac patients often take long naps during the day that are mostly unrefreshing. Researchers found that naps are usually more frequent and longer in those with hypersomnolence than for those in control groups. Furthermore, 75% of those with hypersomnolence reported that short naps are not refreshing, compared to controls.
    2. Chattu, V. K., Sakhamuri, S. M., Kumar, R., Spence, D. W., BaHammam, A. S., & Pandi-Perumal, S. R. (2018). Insufficient Sleep Syndrome: Is it time to classify it as a major noncommunicable disease?. Sleep science (Sao Paulo, Brazil), 11(2), 56–64. https://doi.org/10.5935/1984-0063.20180013↵
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